Cardiac amyloidosis and red flags: natural history and its impact in morbimortality

Abstract

Introduction and objectives

Red flags (RF) are typical cardiac and extracardiac manifestations that may precede the definitive diagnosis of cardiac amiloidosis (CA) by several years, playing a pivotal role in the early diagnosis of the disease. The principal aim of the research was to determine the chronology of onset of RF throughout the natural history of CA as well as its prognostic influence.

Patients and methods

Observational, retrospective inquiry of consecutive patients with a definitive diagnosis of CA in a terciary hospital centre in Ciudad Real (Spain) between February 2016 and December 2023. We defined 21 RF and 3 adverse clinical events, establishing the date of occurrence of each of them.

Results

102 patients (81,6 ± 7,7 years; 84,3% males) with a diagnosis of CA (89,2% TTR; 10,8% AL) were included. The prevalence of RF was very high (8,4 ± 2,3). In the natural history, the first to appear were integumentary, with the most specific cardiological ones being the closest to diagnosis. The 2-year mortality was 49%, with biomarker RFs and the presence of ≥10 RFs being associated with higher mortality.

Conclusions

RFs proved highly prevalent among patients with CA and substantially preceded disease diagnosis. RF burden was associated with prognosis in the follow-up of ATTR patients.